Flashcards in Clinical: Bullous Disease Deck (24):
Pathogenesis of the Immunobullous diseases.
Autoantibodies against skin proteins.
Pathogenesis of Pemphigus Vulgaris (PV)
Antibodies mainly against Desmoglein 3 (interkeratinocyte protein)
Pathogenesis of Pemphigus Foliaceous (PF)
Antibodies against Desmoglein 1 (interkeratinocyte protein)
Differentiating factor of P. Vulgaris vs. Foliaceous on PE
Vulgaris has oral involvement
Foliaceous does not
(also foliaceous looks more eroded thru the skin)
Modality to diagnose Pemphigus diseases.
H&E stain of epidermis
PV: lesional gap between the stratum basalis and the other epidermal layers
PF: stratum corneum shows a focal spot of thinning (erosion)
First line treatment for PV and PF.
Trial monoclonal Ab drug used for PV and PF.
MOA: anti-CD20 antibody
Pemphigus condition associated with Non-Hodgkins lymphoma.
Pathogenesis of Pemphigoid Diseases
Auto Abs against proteins in the dermoepidermal junction
Pathogenesis of Bullous Pemphigoid
Abs against Bullous Pemphigoid Antigen 1 and 2
How are pemphigoid lesions differentiated from pemphigus lesions?
Pemphigus lesions are more flaccid bullae that rupture and because they are more superficial, less scarring occurs.
Pemphigoid lesions are more tense bullae that don't rupture as often but usually scar. Pemphigoid looks much worse than it is and usually can go into remission without treatment
First line treatment for Bullous Pemphigoid
Pathogenesis of Mucous Membrane Pemphigoid
-beta 4 integrin
-alpha 6 integrin
-BPAg2 (Bullous Pemphigus Antigen)
(eye and esophageal ulcers are common)
Treatment for the two types of Mucous Membrane Pemphigoid
Non-scarring: topical corticosteroids, anti-inflammatory meds
Scarring: systemic corticosteroids/cyclophosphamide combo
Serous sequelae of Mucous Membrane Pemphigoid
Pathogenesis of Epidermolysis Bullosa Acquisita
Antibodies against Type VII collagen in the papillary dermal layer.
Forms bullae on acral skin ( finger tips, knuckles, elbows, knees, buttocks, toes, heels, ears)
Treatment for Epidermolysis Bullosa Acquisita
Systemic Corticosteroids, cyclosporine, colchicine, rituximab
Pathogensis of Linear IgA disease
IgA antibodies directed against a malformed skin protein (usually truncated BPAg2)
Most common trigger for Linear IgA disease
Treatment for Linear IgA disease
Stop using vanco
Pathogenesis of Dermatitis Herpetiformis
Skin manifestation of Celiac Sprue (gluten sensitive enteropathy)
Most common location of vesicle lesions of Dermatitis Herpetiformis
First line treatment for Dermatitis Herpetiformis
Initiate Dapsone with a gluten free diet